Glanzmann Thrombasthenia in Children: Experience From a Tertiary Care Center in Southern India

Journal of Pediatric Hematology/oncology
Smitha KongalappaAnand Prakash

Abstract

Glanzmann thrombasthenia (GT) is a globally rare inherited disorder of hemostasis. To describe the clinical profile of GT in a tertiary care center in Southern India. A retrospective chart review of all children with GT was performed between January 2005 and August 2017 in the Department of Paediatrics. A total of 48 patients (representing 43 families) were included. Median age at diagnosis was 2.75 years (interquartile range: 1.5 to 6.75). Two thirds had an onset of bleeding within the first 2 years of life. Sixty-seven percent were born out of consanguineous marriage. The common symptoms were epistaxis, gingival bleeding, and ecchymoses. Neonatal onset of bleeding manifested as purpura, epistaxis, and intracranial hemorrhage. Postsurgical bleeding and menorrhagia were unique presentations in adolescence. About 25% had life-threatening hemorrhage while 50% had growth retardation due to chronic anemia. GT is relatively more common in areas of Southern India due to the higher prevalence of consanguinity. Chronic anemia can contribute to growth stunting in these patients.

References

Apr 15, 1991·Proceedings of the National Academy of Sciences of the United States of America·P J NewmanB S Coller
Mar 1, 1983·Scandinavian Journal of Haematology·A S Awidi
Sep 25, 2004·American Journal of Hematology·G ToogehF Peyvandi
Jul 17, 2013·Journal of Thrombosis and Haemostasis : JTH·A TosettoF Rodeghiero
Sep 1, 2017·Journal of Hematology·Hejer ElmahmoudiEmna Gouider

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Citations

Jul 17, 2021·Indian Journal of Hematology & Blood Transfusion : an Official Journal of Indian Society of Hematology and Blood Transfusion·Ritika SharmaReena Das

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Methods Mentioned

BETA
flow cytometry
contraception

Software Mentioned

Statistical Package for Social Sciences ( SPSS )

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